HNPP: A Rare Cause of Entrapment Neuropathies in Young Adults

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Hereditary Neuropathy with Liability to Pressure Palsies in a Turkish

Patient (HNPP): A Rare Cause of Entrapment Neuropathies in Young

Adults.

Celýk Y, Kilinçer C, Hamamcioðlu MK, Balci K, Býrgýlý B, Cobanoðlu S,

Utku U.

Trakya University School of Medicine Neurology, Edirne, Turkey.

Hereditary neuropathy with liability to pressure palsies (HNPP) is an

autosomal dominant nerve disease usually caused by 1,5 Mb deletion on

chromosome 17p11.2.2-p12, the region where the PMP-22 gene is

located. The patients with HNPP usually have relapsing and remitting

entrapment neuropathies due to compression.

We present a 14-year-old male who had acute onset, right-sided ulnar

nerve entrapment at the elbow. He had electrophysiological findings

of bilateral ulnar nerve entrapments (more severe at the right side)

at the elbow and bilateral median nerve entrapment at the wrist.

Genetic tests of the patient demonstrated deletions in the 17p11.2

region.

The patient underwent decompressive surgery for ulnar nerve

entrapment at the elbow and completely recovered two months after the

event. Although HNPP is extremely rare, it should be taken into

consideration in young adults with entrapment neuropathies.